Define
- Symptoms of chronic hydrocephalus, a head circumference of more than 2 standard deviations above the 98th percentile and overt tri-ventriculomegaly on neuroimaging, in the absence of a secondary cause for aqueductal stenosis in adulthood.
Pathophysiology (unclear)
- Full or partial obstruction of CSF flow through the aqueduct of Sylvius before fusion of cranial sutures (i.e. before age 24 months) → restoration of CSF flow before clinical symptoms can manifest in childhood (arrested Hydrocephalus).
- During the arrested hydrocephalic period CSF flow is likely maintained via a combination of
- Recanalization of the aqueduct,
- Utilisation of alternative CSF flow routes,
- Modification of CSF production or absorption
- Reversal of arrested HCP to a symptomatic one by the following mechanism
- Failure of adequate CSF flow
- Skull base changes as a consequence of chronic and progressive rise in ICP
- Chronic pressure effects upon brain parenchyma
Diagnostic criteria
- Clinical symptoms of hydrocephalus developing in adulthood
- e.g. Headaches, cognitive decline, imbalance, gait disturbance, psychological disturbance, visual deterioration/diplopia
- Macrocephaly
- Defined by head circumference >98ᵗʰ percentile in adulthood (male 53.8 cm; female 52.9 cm)
- Imaging
- Overt tri-ventriculomegaly (lateral and third ventricles) on neuroimaging, with cortical sulcal effacement and/or destruction of the sella turcica as evidence of long-standing ventriculomegaly
- Absence of a secondary cause for aqueductal stenosis in adulthood (e.g. previous meningitis, subarachnoid haemorrhage)
Clinical features
- Headaches
- Imbalance
- Cognitive and psychological problems
- Decline in memory,
- Decline in attention and language skills
- Along with depression
- Anxiety and disinhibition
Radiology
- Triventriculomegaly with obliteration of septum pellucidium
- D) Subtle sulcal effacement may be appreciated, particularly in the right temporo-parietal region,
Treatment
- Shunt
- ETV
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